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Hereditary spherocytosis HY

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Rickettsia Rickettsii
Rickettsia Rickettsii

Hereditary spherocytosis

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Hereditary spherocytosis Epidemiology • Usually autosomal dominant • Northern European descent Clinical presentation • Hemolytic anemia • Jaundice • Splenomegaly Laboratory findings • ↑ MCHC • Negative Coombs test • Spherocytes on peripheral smear • ↑ Osmotic fragility on acidified glycerol lysis test • Abnormal eosin-5-maleimide binding test Treatment • Folic acid supplementation • Blood transfusion • Splenectomy

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Hereditary spherocytosis HY

  • 1. Hereditary spherocytosis Epidemiology • Usually autosomal dominant • Northern European descent Clinical presentation • Hemolytic anemia • Jaundice • Splenomegaly Laboratory findings • ↑ MCHC • Negative Coombs test • Spherocytes on peripheral smear • ↑ Osmotic fragility on acidified glycerol lysis test • Abnormal eosin-5-maleimide binding test Treatment • Folic acid supplementation • Blood transfusion • Splenectomy